Idiopathic Pulmonary Fibrosis Flashcards
What is IPF?
Interstitial lung disease of
Imbalance of fibrotic repair system of unknown origin
Some query the relation of IPF to?
Gastric reflux
What are secondary causes of IPF?
Rheumatoid SLE Asbestos Systemic sclerosis Drugs
Which drugs are secondary causes of IPF?
Amiodarone Busulphan Bleomycin Penicillamine Nitrofurantoin Methotrexate
What are symptoms of IPF?
Progressive dyspnoea (over years) - worse on exertion
Dry cough
Weight loss
Arthralgia
What are signs of IPF?
Cyanosis
Finger clubbing
Fine-end inspiratory crepitations
Which investigations can be performed for IPF?
Bloods
CXR
Spirometry
Which blood results would be expected in an IPF patient?
Decreased PaO2 increased PaCO2 (if severe) Increased CRP Increased Immunoglobulins (rheumatoid factor 10% +ve)
What is expected to be seen on a CXR of an IPF patient?
Decreased lung volume
Bilateral lower zone reticulo-nodular shadows
Traction bronchiectasis
Honeycomb lung (advanced disease)
What kind of spirometry would IPF show?
Restrictive
What are possible complications of IPF?
Respiratory failure
Increased risk of lung cancer
What type of pathology is associated with IPF`
Usual interstitial pneumonia pattern
Heterogenous fibres fibrosis in alveolar walls with fibroblastic foci and destruction of architecture causing honeycombing
Inflammation is minimal
How do you treat IPF?
Antifibrotic drugs (slow progression but dines't reverse are in clinical trials= Palliative care Opiates Oxygen if hypoxia Pulmonary rehabilitation
All IPF patients should be considered for?
Transplant
Clinical trials
What would be seen on a CT scan of IPF?
Reticulonodular fibrotic changes (worse at bases -irreversible)
“Ground glass” shows alveolitis (reversible)
